Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study
Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT)...
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| Veröffentlicht in: | Hepatology (Baltimore, Md.) Md.), 2020-02, Vol.71 (2), p.658-669 |
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| creator | Baiges, Anna Turon, Fanny Simón‐Talero, Macarena Tasayco, Stephanie Bueno, Javier Zekrini, Kamal Plessier, Aurélie Franchi‐Abella, Stéphanie Guerin, Florent Mukund, Amar Eapen, C.E. Goel, Ashish Shyamkumar, Nidugala Keshava Coenen, Sandra Gottardi, Andrea Majumdar, Avik Onali, Simona Shukla, Akash Carrilho, Flair José Nacif, Lucas Primignani, Massimo Tosetti, Giulia Mura, Vicenzo Nevens, Frederik Witters, Peter Tripathi, Dhiraj Tellez, Luis Martínez, Javier Álvarez‐Navascués, Carmen Fraile López, Miguel López Procopet, Bogdan Piscaglia, Fabio Koning, Barbara Llop, Elba Romero‐Cristobal, Mario Tjwa, Eric Monescillo‐Francia, Alberto Senzolo, Marco Perez‐LaFuente, Mercedes Segarra, Antonio Sarin, Shiv Kumar Hernández‐Gea, Virginia Patch, David Laleman, Wim Hartog, Hermien Valla, Dominique Genescà, Joan García‐Pagán, Juan Carlos |
| description | Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty‐six patients were included; median age at the end of follow‐up was 30 years. Nineteen patients (28%) presented HE. Ten‐, 20‐, and 30‐year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty‐five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach. |
| doi_str_mv | 10.1002/hep.30817 |
| format | Article |
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Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty‐six patients were included; median age at the end of follow‐up was 30 years. Nineteen patients (28%) presented HE. Ten‐, 20‐, and 30‐year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty‐five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.</description><identifier>ISSN: 0270-9139</identifier><identifier>EISSN: 1527-3350</identifier><identifier>DOI: 10.1002/hep.30817</identifier><identifier>PMID: 31211875</identifier><language>eng</language><publisher>United States: Wolters Kluwer Health, Inc</publisher><subject>Adenoma ; Adolescent ; Adult ; Age ; Aged ; Child ; Child, Preschool ; Dyspnea ; Female ; Hepatic encephalopathy ; Hepatic Encephalopathy - epidemiology ; Hepatic Encephalopathy - etiology ; Hepatocellular carcinoma ; Hepatology ; Hepatopulmonary Syndrome - epidemiology ; Hepatopulmonary Syndrome - etiology ; Humans ; Hypertension, Pulmonary - epidemiology ; Hypertension, Pulmonary - etiology ; Infant ; International Cooperation ; Liver cancer ; Liver Neoplasms - epidemiology ; Liver Neoplasms - etiology ; Liver transplantation ; Male ; Middle Aged ; Observational studies ; Portal Vein - abnormalities ; Respiration ; Retrospective Studies ; Shunts ; Tumors ; Vascular Malformations - complications ; Vascular Malformations - diagnosis ; Young Adult</subject><ispartof>Hepatology (Baltimore, Md.), 2020-02, Vol.71 (2), p.658-669</ispartof><rights>2019 by the American Association for the Study of Liver Diseases.</rights><rights>2020 by the American Association for the Study of Liver Diseases.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4547-1dcdfada937a2ce494a700e56b1bc039008ca0d3a10a48e07be606911f312b6a3</citedby><cites>FETCH-LOGICAL-c4547-1dcdfada937a2ce494a700e56b1bc039008ca0d3a10a48e07be606911f312b6a3</cites><orcidid>0000-0002-0544-5610 ; 0000-0002-0831-8422 ; 0000-0001-9032-4954 ; 0000-0001-6656-5387</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fhep.30817$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fhep.30817$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31211875$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Baiges, Anna</creatorcontrib><creatorcontrib>Turon, Fanny</creatorcontrib><creatorcontrib>Simón‐Talero, Macarena</creatorcontrib><creatorcontrib>Tasayco, Stephanie</creatorcontrib><creatorcontrib>Bueno, Javier</creatorcontrib><creatorcontrib>Zekrini, Kamal</creatorcontrib><creatorcontrib>Plessier, Aurélie</creatorcontrib><creatorcontrib>Franchi‐Abella, Stéphanie</creatorcontrib><creatorcontrib>Guerin, Florent</creatorcontrib><creatorcontrib>Mukund, Amar</creatorcontrib><creatorcontrib>Eapen, C.E.</creatorcontrib><creatorcontrib>Goel, Ashish</creatorcontrib><creatorcontrib>Shyamkumar, Nidugala Keshava</creatorcontrib><creatorcontrib>Coenen, Sandra</creatorcontrib><creatorcontrib>Gottardi, Andrea</creatorcontrib><creatorcontrib>Majumdar, Avik</creatorcontrib><creatorcontrib>Onali, Simona</creatorcontrib><creatorcontrib>Shukla, Akash</creatorcontrib><creatorcontrib>Carrilho, Flair José</creatorcontrib><creatorcontrib>Nacif, Lucas</creatorcontrib><creatorcontrib>Primignani, Massimo</creatorcontrib><creatorcontrib>Tosetti, Giulia</creatorcontrib><creatorcontrib>Mura, Vicenzo</creatorcontrib><creatorcontrib>Nevens, Frederik</creatorcontrib><creatorcontrib>Witters, Peter</creatorcontrib><creatorcontrib>Tripathi, Dhiraj</creatorcontrib><creatorcontrib>Tellez, Luis</creatorcontrib><creatorcontrib>Martínez, Javier</creatorcontrib><creatorcontrib>Álvarez‐Navascués, Carmen</creatorcontrib><creatorcontrib>Fraile López, Miguel López</creatorcontrib><creatorcontrib>Procopet, Bogdan</creatorcontrib><creatorcontrib>Piscaglia, Fabio</creatorcontrib><creatorcontrib>Koning, Barbara</creatorcontrib><creatorcontrib>Llop, Elba</creatorcontrib><creatorcontrib>Romero‐Cristobal, Mario</creatorcontrib><creatorcontrib>Tjwa, Eric</creatorcontrib><creatorcontrib>Monescillo‐Francia, Alberto</creatorcontrib><creatorcontrib>Senzolo, Marco</creatorcontrib><creatorcontrib>Perez‐LaFuente, Mercedes</creatorcontrib><creatorcontrib>Segarra, Antonio</creatorcontrib><creatorcontrib>Sarin, Shiv Kumar</creatorcontrib><creatorcontrib>Hernández‐Gea, Virginia</creatorcontrib><creatorcontrib>Patch, David</creatorcontrib><creatorcontrib>Laleman, Wim</creatorcontrib><creatorcontrib>Hartog, Hermien</creatorcontrib><creatorcontrib>Valla, Dominique</creatorcontrib><creatorcontrib>Genescà, Joan</creatorcontrib><creatorcontrib>García‐Pagán, Juan Carlos</creatorcontrib><creatorcontrib>REHEVASC, VALDIG an EASL consortium, Abernethy group</creatorcontrib><creatorcontrib>for the REHEVASC, VALDIG an EASL consortium, Abernethy group</creatorcontrib><title>Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study</title><title>Hepatology (Baltimore, Md.)</title><addtitle>Hepatology</addtitle><description>Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty‐six patients were included; median age at the end of follow‐up was 30 years. Nineteen patients (28%) presented HE. Ten‐, 20‐, and 30‐year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty‐five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.</description><subject>Adenoma</subject><subject>Adolescent</subject><subject>Adult</subject><subject>Age</subject><subject>Aged</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Dyspnea</subject><subject>Female</subject><subject>Hepatic encephalopathy</subject><subject>Hepatic Encephalopathy - epidemiology</subject><subject>Hepatic Encephalopathy - etiology</subject><subject>Hepatocellular carcinoma</subject><subject>Hepatology</subject><subject>Hepatopulmonary Syndrome - epidemiology</subject><subject>Hepatopulmonary Syndrome - etiology</subject><subject>Humans</subject><subject>Hypertension, Pulmonary - epidemiology</subject><subject>Hypertension, Pulmonary - etiology</subject><subject>Infant</subject><subject>International Cooperation</subject><subject>Liver cancer</subject><subject>Liver Neoplasms - epidemiology</subject><subject>Liver Neoplasms - etiology</subject><subject>Liver transplantation</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Observational studies</subject><subject>Portal Vein - abnormalities</subject><subject>Respiration</subject><subject>Retrospective Studies</subject><subject>Shunts</subject><subject>Tumors</subject><subject>Vascular Malformations - complications</subject><subject>Vascular Malformations - diagnosis</subject><subject>Young Adult</subject><issn>0270-9139</issn><issn>1527-3350</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kcFKw0AQhhdRtFYPvoAEvNRD6uxu0mS9lVK1oFRQz2GTTGxqkq27GzVv72qsB8HTMDMfH8w_hJxQGFMAdrHCzZhDTKMdMqAhi3zOQ9glA2AR-IJycUAOjVkDgAhYvE8OOGWUxlE4IC8z1TxjU1pZefMPq6VzSVtm3r3SVpnOWKxd97BqG2u80TRF3aBddd6drAqla8eq5vzSmzbeorFu-T1wsmVqUL9tuwfb5t0R2StkZfD4pw7J09X8cXbj3y6vF7PprZ8FYRD5NM_yQuZS8EiyDAMRyAgAw0lK0wy4AIgzCTmXFGQQI0QpTmAiKC3cWelE8iEZ9d6NVq8tGpvUpcmwqmSDqjUJYwEPREwpOPTsD7pWrTuichQPWchjJoSjznsq08oYjUWy0WUtdZdQSL4-kLjUku8POPb0x9imNea_5DZyB1z0wHtZYfe_KbmZ3_fKT_xakGc</recordid><startdate>202002</startdate><enddate>202002</enddate><creator>Baiges, Anna</creator><creator>Turon, Fanny</creator><creator>Simón‐Talero, Macarena</creator><creator>Tasayco, Stephanie</creator><creator>Bueno, Javier</creator><creator>Zekrini, Kamal</creator><creator>Plessier, Aurélie</creator><creator>Franchi‐Abella, Stéphanie</creator><creator>Guerin, Florent</creator><creator>Mukund, Amar</creator><creator>Eapen, C.E.</creator><creator>Goel, Ashish</creator><creator>Shyamkumar, Nidugala Keshava</creator><creator>Coenen, Sandra</creator><creator>Gottardi, Andrea</creator><creator>Majumdar, Avik</creator><creator>Onali, Simona</creator><creator>Shukla, Akash</creator><creator>Carrilho, Flair José</creator><creator>Nacif, Lucas</creator><creator>Primignani, Massimo</creator><creator>Tosetti, Giulia</creator><creator>Mura, Vicenzo</creator><creator>Nevens, Frederik</creator><creator>Witters, Peter</creator><creator>Tripathi, Dhiraj</creator><creator>Tellez, Luis</creator><creator>Martínez, Javier</creator><creator>Álvarez‐Navascués, Carmen</creator><creator>Fraile López, Miguel López</creator><creator>Procopet, Bogdan</creator><creator>Piscaglia, Fabio</creator><creator>Koning, Barbara</creator><creator>Llop, Elba</creator><creator>Romero‐Cristobal, Mario</creator><creator>Tjwa, Eric</creator><creator>Monescillo‐Francia, Alberto</creator><creator>Senzolo, Marco</creator><creator>Perez‐LaFuente, Mercedes</creator><creator>Segarra, Antonio</creator><creator>Sarin, Shiv Kumar</creator><creator>Hernández‐Gea, Virginia</creator><creator>Patch, David</creator><creator>Laleman, Wim</creator><creator>Hartog, Hermien</creator><creator>Valla, Dominique</creator><creator>Genescà, Joan</creator><creator>García‐Pagán, Juan Carlos</creator><general>Wolters Kluwer Health, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TM</scope><scope>7TO</scope><scope>7U9</scope><scope>H94</scope><scope>K9.</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-0544-5610</orcidid><orcidid>https://orcid.org/0000-0002-0831-8422</orcidid><orcidid>https://orcid.org/0000-0001-9032-4954</orcidid><orcidid>https://orcid.org/0000-0001-6656-5387</orcidid></search><sort><creationdate>202002</creationdate><title>Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study</title><author>Baiges, Anna ; Turon, Fanny ; Simón‐Talero, Macarena ; Tasayco, Stephanie ; Bueno, Javier ; Zekrini, Kamal ; Plessier, Aurélie ; Franchi‐Abella, Stéphanie ; Guerin, Florent ; Mukund, Amar ; Eapen, C.E. ; Goel, Ashish ; Shyamkumar, Nidugala Keshava ; Coenen, Sandra ; Gottardi, Andrea ; Majumdar, Avik ; Onali, Simona ; Shukla, Akash ; Carrilho, Flair José ; Nacif, Lucas ; Primignani, Massimo ; Tosetti, Giulia ; Mura, Vicenzo ; Nevens, Frederik ; Witters, Peter ; Tripathi, Dhiraj ; Tellez, Luis ; Martínez, Javier ; Álvarez‐Navascués, Carmen ; Fraile López, Miguel López ; Procopet, Bogdan ; Piscaglia, Fabio ; Koning, Barbara ; Llop, Elba ; Romero‐Cristobal, Mario ; Tjwa, Eric ; Monescillo‐Francia, Alberto ; Senzolo, Marco ; Perez‐LaFuente, Mercedes ; Segarra, Antonio ; Sarin, Shiv Kumar ; Hernández‐Gea, Virginia ; Patch, David ; Laleman, Wim ; Hartog, Hermien ; Valla, Dominique ; Genescà, Joan ; García‐Pagán, Juan Carlos</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4547-1dcdfada937a2ce494a700e56b1bc039008ca0d3a10a48e07be606911f312b6a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Adenoma</topic><topic>Adolescent</topic><topic>Adult</topic><topic>Age</topic><topic>Aged</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Dyspnea</topic><topic>Female</topic><topic>Hepatic encephalopathy</topic><topic>Hepatic Encephalopathy - epidemiology</topic><topic>Hepatic Encephalopathy - etiology</topic><topic>Hepatocellular carcinoma</topic><topic>Hepatology</topic><topic>Hepatopulmonary Syndrome - epidemiology</topic><topic>Hepatopulmonary Syndrome - etiology</topic><topic>Humans</topic><topic>Hypertension, Pulmonary - epidemiology</topic><topic>Hypertension, Pulmonary - etiology</topic><topic>Infant</topic><topic>International Cooperation</topic><topic>Liver cancer</topic><topic>Liver Neoplasms - epidemiology</topic><topic>Liver Neoplasms - etiology</topic><topic>Liver transplantation</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Observational studies</topic><topic>Portal Vein - abnormalities</topic><topic>Respiration</topic><topic>Retrospective Studies</topic><topic>Shunts</topic><topic>Tumors</topic><topic>Vascular Malformations - complications</topic><topic>Vascular Malformations - diagnosis</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Baiges, Anna</creatorcontrib><creatorcontrib>Turon, Fanny</creatorcontrib><creatorcontrib>Simón‐Talero, Macarena</creatorcontrib><creatorcontrib>Tasayco, Stephanie</creatorcontrib><creatorcontrib>Bueno, Javier</creatorcontrib><creatorcontrib>Zekrini, Kamal</creatorcontrib><creatorcontrib>Plessier, Aurélie</creatorcontrib><creatorcontrib>Franchi‐Abella, Stéphanie</creatorcontrib><creatorcontrib>Guerin, Florent</creatorcontrib><creatorcontrib>Mukund, Amar</creatorcontrib><creatorcontrib>Eapen, C.E.</creatorcontrib><creatorcontrib>Goel, Ashish</creatorcontrib><creatorcontrib>Shyamkumar, Nidugala Keshava</creatorcontrib><creatorcontrib>Coenen, Sandra</creatorcontrib><creatorcontrib>Gottardi, Andrea</creatorcontrib><creatorcontrib>Majumdar, Avik</creatorcontrib><creatorcontrib>Onali, Simona</creatorcontrib><creatorcontrib>Shukla, Akash</creatorcontrib><creatorcontrib>Carrilho, Flair José</creatorcontrib><creatorcontrib>Nacif, Lucas</creatorcontrib><creatorcontrib>Primignani, Massimo</creatorcontrib><creatorcontrib>Tosetti, Giulia</creatorcontrib><creatorcontrib>Mura, Vicenzo</creatorcontrib><creatorcontrib>Nevens, Frederik</creatorcontrib><creatorcontrib>Witters, Peter</creatorcontrib><creatorcontrib>Tripathi, Dhiraj</creatorcontrib><creatorcontrib>Tellez, Luis</creatorcontrib><creatorcontrib>Martínez, Javier</creatorcontrib><creatorcontrib>Álvarez‐Navascués, Carmen</creatorcontrib><creatorcontrib>Fraile López, Miguel López</creatorcontrib><creatorcontrib>Procopet, Bogdan</creatorcontrib><creatorcontrib>Piscaglia, Fabio</creatorcontrib><creatorcontrib>Koning, Barbara</creatorcontrib><creatorcontrib>Llop, Elba</creatorcontrib><creatorcontrib>Romero‐Cristobal, Mario</creatorcontrib><creatorcontrib>Tjwa, Eric</creatorcontrib><creatorcontrib>Monescillo‐Francia, Alberto</creatorcontrib><creatorcontrib>Senzolo, Marco</creatorcontrib><creatorcontrib>Perez‐LaFuente, Mercedes</creatorcontrib><creatorcontrib>Segarra, Antonio</creatorcontrib><creatorcontrib>Sarin, Shiv Kumar</creatorcontrib><creatorcontrib>Hernández‐Gea, Virginia</creatorcontrib><creatorcontrib>Patch, David</creatorcontrib><creatorcontrib>Laleman, Wim</creatorcontrib><creatorcontrib>Hartog, Hermien</creatorcontrib><creatorcontrib>Valla, Dominique</creatorcontrib><creatorcontrib>Genescà, Joan</creatorcontrib><creatorcontrib>García‐Pagán, Juan Carlos</creatorcontrib><creatorcontrib>REHEVASC, VALDIG an EASL consortium, Abernethy group</creatorcontrib><creatorcontrib>for the REHEVASC, VALDIG an EASL consortium, Abernethy group</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>Hepatology (Baltimore, Md.)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Baiges, Anna</au><au>Turon, Fanny</au><au>Simón‐Talero, Macarena</au><au>Tasayco, Stephanie</au><au>Bueno, Javier</au><au>Zekrini, Kamal</au><au>Plessier, Aurélie</au><au>Franchi‐Abella, Stéphanie</au><au>Guerin, Florent</au><au>Mukund, Amar</au><au>Eapen, C.E.</au><au>Goel, Ashish</au><au>Shyamkumar, Nidugala Keshava</au><au>Coenen, Sandra</au><au>Gottardi, Andrea</au><au>Majumdar, Avik</au><au>Onali, Simona</au><au>Shukla, Akash</au><au>Carrilho, Flair José</au><au>Nacif, Lucas</au><au>Primignani, Massimo</au><au>Tosetti, Giulia</au><au>Mura, Vicenzo</au><au>Nevens, Frederik</au><au>Witters, Peter</au><au>Tripathi, Dhiraj</au><au>Tellez, Luis</au><au>Martínez, Javier</au><au>Álvarez‐Navascués, Carmen</au><au>Fraile López, Miguel López</au><au>Procopet, Bogdan</au><au>Piscaglia, Fabio</au><au>Koning, Barbara</au><au>Llop, Elba</au><au>Romero‐Cristobal, Mario</au><au>Tjwa, Eric</au><au>Monescillo‐Francia, Alberto</au><au>Senzolo, Marco</au><au>Perez‐LaFuente, Mercedes</au><au>Segarra, Antonio</au><au>Sarin, Shiv Kumar</au><au>Hernández‐Gea, Virginia</au><au>Patch, David</au><au>Laleman, Wim</au><au>Hartog, Hermien</au><au>Valla, Dominique</au><au>Genescà, Joan</au><au>García‐Pagán, Juan Carlos</au><aucorp>REHEVASC, VALDIG an EASL consortium, Abernethy group</aucorp><aucorp>for the REHEVASC, VALDIG an EASL consortium, Abernethy group</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study</atitle><jtitle>Hepatology (Baltimore, Md.)</jtitle><addtitle>Hepatology</addtitle><date>2020-02</date><risdate>2020</risdate><volume>71</volume><issue>2</issue><spage>658</spage><epage>669</epage><pages>658-669</pages><issn>0270-9139</issn><eissn>1527-3350</eissn><abstract>Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty‐six patients were included; median age at the end of follow‐up was 30 years. Nineteen patients (28%) presented HE. Ten‐, 20‐, and 30‐year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty‐five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.</abstract><cop>United States</cop><pub>Wolters Kluwer Health, Inc</pub><pmid>31211875</pmid><doi>10.1002/hep.30817</doi><tpages>12</tpages><orcidid>https://orcid.org/0000-0002-0544-5610</orcidid><orcidid>https://orcid.org/0000-0002-0831-8422</orcidid><orcidid>https://orcid.org/0000-0001-9032-4954</orcidid><orcidid>https://orcid.org/0000-0001-6656-5387</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0270-9139 |
| ispartof | Hepatology (Baltimore, Md.), 2020-02, Vol.71 (2), p.658-669 |
| issn | 0270-9139 1527-3350 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_2243498110 |
| source | MEDLINE; Wiley Online Library Journals Frontfile Complete; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals |
| subjects | Adenoma Adolescent Adult Age Aged Child Child, Preschool Dyspnea Female Hepatic encephalopathy Hepatic Encephalopathy - epidemiology Hepatic Encephalopathy - etiology Hepatocellular carcinoma Hepatology Hepatopulmonary Syndrome - epidemiology Hepatopulmonary Syndrome - etiology Humans Hypertension, Pulmonary - epidemiology Hypertension, Pulmonary - etiology Infant International Cooperation Liver cancer Liver Neoplasms - epidemiology Liver Neoplasms - etiology Liver transplantation Male Middle Aged Observational studies Portal Vein - abnormalities Respiration Retrospective Studies Shunts Tumors Vascular Malformations - complications Vascular Malformations - diagnosis Young Adult |
| title | Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study |
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